STEVENS-JOHNSON-SYNDROME (SJS)

Stevens-Johnson Syndrome, often referred to as SJS, is a rare yet grave condition that warrants attention. Typically triggered by certain medications, it initiates a severe reaction within your body. The hallmark of SJS is the blistering and peeling of your skin, accompanied by an impact on your mucous membranes. This reaction doesn’t just affect the surface; it extends inside your body, complicating basic functions such as eating, swallowing, and even urinating.

Alternative names for Stevens-Johnson syndrome include Lyell’s syndrome, Stevens-Johnson syndrome/toxic epidermal necrolysis, and Stevens-Johnson syndrome toxic epidermal necrolysis spectrum. It may also be referred to as drug-induced Stevens-Johnson syndrome or mycoplasma-induced Stevens-Johnson syndrome if linked to a specific cause.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe skin conditions characterized by the development of rashes, blisters, and subsequent peeling of the skin. These conditions also affect mucous membranes, including the eyes, genitalia, and mouth. Individuals afflicted with SJS or TEN typically require hospitalization due to the seriousness of the condition.

While some view SJS and TEN as distinct diseases, others perceive them as different severities of the same condition. SJS is generally considered less severe than TEN; for example, skin peeling may affect less than 10% of the body in SJS compared to over 30% in TEN. However, both conditions can pose life-threatening risks.

Immediate medical intervention is crucial as it can safeguard your skin and prevent lasting harm to other vital organs.

Understanding the Differences between SJS and TEN:

Stevens-Johnson Syndrome (SJS) bears similarities to another condition known as toxic epidermal necrolysis (TEN). Both conditions involve the shedding of skin and often stem from an immune system reaction to medications. However, TEN can also be triggered by factors like infections, tumors, or vaccines.

The primary disparity between SJS and TEN lies in their scale of impact. SJS typically affects 10% or less of the body’s surface, while TEN extends to 30% or more.

TEN poses a severe threat to life. Approximately 25% of TEN cases result in fatality. On the other hand, SJS is comparatively less lethal, with a fatality rate ranging between 1% and 5%.

Causes of Stevens-Johnson Syndrome

Several factors can contribute to the onset of Stevens-Johnson Syndrome. Over 100 medications have been linked to SJS, with some of the most common culprits including:

Medications for gout, notably allopurinol (Aloprim, Zyloprim)
Pain relievers like acetaminophen, ibuprofen, and naproxen sodium
Sulfa antibiotics, used to combat infections (e.g., Bactrim and Septra)
Drugs used to treat seizures or mental health disorders

In children, sulfa antibiotics, Tylenol, and seizure medications, particularly carbamazepine (Carbatrol, Tegretol), are more likely to cause issues.

It’s worth noting that if SJS occurs, it typically manifests within the first two months of starting a new medication. Infections such as pneumonia or the herpes virus, especially in children, can also act as triggers for SJS. Additionally, conditions like graft-versus-host disease following a stem cell transplant can lead to SJS.

Certain predisposing factors increase the likelihood of developing SJS, including:

HIV or other immune system disorders like lupus
Previous occurrences of SJS
Specific genetic variations, particularly in the human leukocyte antigen-B gene inherited from parents
History of radiation therapy or bone marrow transplants

In some cases, the exact cause of Stevens-Johnson Syndrome remains elusive even after medical evaluation.

Symptoms of Stevens-Johnson Syndrome

Symptoms of Stevens-Johnson Syndrome (SJS) often begin with flu-like symptoms such as fever and a general feeling of being unwell. After a few days, additional symptoms emerge, including:

Painful, reddened skin resembling burns, which eventually peels off
Blisters appearing on the skin, mouth, nose, and genital areas
Red, watery, and painful eyes

The rash associated with SJS is sometimes likened to a target, characterized by concentric circles with lighter edges and a darker center. It’s crucial to recognize that SJS is a serious condition, and if you experience these symptoms, seeking immediate medical attention at the emergency room is imperative.

Risk factors

The risk factors for developing Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are multifaceted, involving both genetic and environmental influences. Here’s a breakdown of the key risk factors:

Genetic Predisposition: There is evidence to suggest that individuals with certain genetic variations may be more susceptible to developing SJS or TEN. Specific human leukocyte antigens (HLAs), which play a crucial role in immune system function, have been identified as genetic factors that can increase the risk of these disorders.
Environmental Triggers: While genetic predisposition sets the stage for SJS or TEN, environmental factors often act as triggers that initiate the onset of the condition. These triggers can include various medications, infections (such as pneumonia or herpes), and other external factors.
Medications: The most common trigger for SJS and TEN is the use of certain medications. These can include antibiotics (such as sulfonamides), anticonvulsants (like carbamazepine and phenytoin), nonsteroidal anti-inflammatory drugs (NSAIDs), and drugs used to treat infections or mental health disorders.
Infections: In some cases, infections such as pneumonia or viral infections like herpes can precipitate the development of SJS or TEN, particularly in children.
Age and Gender: While SJS and TEN can occur at any age, they are more commonly seen in children and young adults under the age of 30. Additionally, there appears to be a slightly higher incidence of these conditions in females compared to males.

Diagnosing Stevens-Johnson Syndrome

Diagnosing Stevens-Johnson Syndrome (SJS) involves a thorough examination by your healthcare provider, focusing on your medical history and recent medication use. Here’s what you can anticipate during the diagnostic process:

Medical History Assessment: Your healthcare provider will inquire about your medical history, paying particular attention to any recent medications you’ve started or stopped taking. This information helps identify potential triggers for SJS.
Rash Examination: Your healthcare provider will visually inspect the rash associated with SJS. This examination is crucial for initial assessment and may prompt further diagnostic steps.
Additional Tests:

Skin Biopsy: A small sample of your skin will be removed for examination under a microscope. This biopsy helps confirm the diagnosis of SJS and assess the severity of skin involvement.
Culture: Skin, tissue, or fluid samples may be collected to rule out infections that could be contributing to your condition.
Imaging: If pneumonia is suspected based on your symptoms, imaging tests of your lungs may be performed to evaluate their condition.
Blood Test: A blood sample will be taken to detect any signs of infection or other abnormalities that could be relevant to your condition.

Pain Assessment: Your healthcare team will assess your pain level, which is an important aspect of monitoring and managing SJS.
Rash Progression and Skin Involvement: Observing how quickly the rash spreads and determining the extent of skin affected are critical factors in evaluating the severity of SJS.

Treating Stevens-Johnson Syndrome

Treatment for Stevens-Johnson Syndrome (SJS) typically occurs in a hospital setting, overseen by a specialized team of healthcare professionals. In some cases, individuals may receive care in a burn center or intensive care unit for optimal management. Here’s what you can expect during SJS treatment:

Addressing Underlying Causes: The foremost priority is to discontinue the medication responsible for triggering SJS or to treat any underlying infection contributing to the condition. This proactive approach helps halt further progression and supports the body’s healing process.
Symptom Management: Healthcare providers focus on alleviating symptoms, preventing infections, and facilitating the healing process. This may involve:
- Fluid and Nutrient Replacement: Maintaining hydration and supplying essential nutrients are crucial for supporting the body’s recovery. Initially, fluids may be administered intravenously (IV), followed by enteral feeding through a tube inserted into the stomach via the nose.
- Wound Care: Diligent wound care is essential to prevent infection and promote healing. Hospital staff delicately cleanse the skin, remove dead tissue, and apply specialized dressings to protect exposed areas.
- Eye Care: Special attention is given to eye care to prevent dryness and complications. Your healthcare team will cleanse your eyes and administer specific drops and creams to maintain moisture and protect against damage.
Hospital Stay Duration: The duration of hospitalization for SJS treatment typically ranges from 2 to 4 weeks. Recovery from SJS is gradual, and most individuals eventually overcome the condition with appropriate medical care and support.
Long-Term Effects: It’s important to note that SJS may leave lasting effects that manifest years after recovery, including:
- Scarring: Residual scars may remain where the skin peeled during the acute phase of SJS.
- Oral Health Issues: Infections in the gums or mouth can occur as a consequence of SJS.
- Respiratory Complications: Lung problems such as bronchitis, characterized by a persistent cough and difficulty breathing, may develop as a long-term consequence of SJS.

While recovery from SJS is feasible, ongoing monitoring and management are necessary to address potential long-term effects and ensure optimal health outcomes.

Recovery process

Recovery from SJS typically involves a gradual healing process:

Skin Healing: Unless complicated by an infection, skin healing typically begins within 2 to 3 weeks.
Fatigue: It’s common to experience fatigue after leaving the hospital, and it may take weeks or even months before feeling fully recovered.
Medication Avoidance: If a medication triggered SJS, it’s crucial to avoid it permanently to prevent recurrence.

Stevens-Johnson Syndrome Complications

Complications of Stevens-Johnson Syndrome (SJS) can be severe and may include:

Eye Problems: These may manifest as dry eyes, light sensitivity, and long-term vision issues.
Respiratory Complications: Pneumonia, shock, and sepsis, characterized by a systemic inflammatory response to infection, can occur.
Multiple Organ Failure: In the most critical cases, SJS can lead to multiple organ failure, affecting vital organs such as the liver, kidneys, heart, brain, lungs, and small intestine. However, this outcome is relatively rare.

After recovering from SJS, individuals may experience longer-term issues such as:

Increased Sweating
Dry or Itchy Skin
Hair Loss
Nail Abnormalities or Loss
Changes in Taste Perception
Dry Mucous Membranes, leading to difficulties in urination
Chronic Fatigue
Respiratory Problems like asthma or COPD

While SJS recovery can be challenging and may entail long-term effects, with appropriate medical care and support, many individuals eventually regain their health and quality of life.

Preventing Stevens-Johnson Syndrome

Preventing Stevens-Johnson Syndrome (SJS) can be challenging due to the unpredictable nature of individual reactions to medications. However, there are some steps you can take to reduce your risk:

Genetic Testing: If you’re of Asian descent, you may have a gene that increases your susceptibility to SJS, particularly when taking carbamazepine. Consider discussing genetic testing with your doctor before starting this medication.
Prior SJS History: If you’ve previously experienced SJS, take proactive measures to prevent recurrence:
- Inform your healthcare providers about your SJS history.
- Wear a medical alert bracelet to alert medical personnel in case of emergency.
- Avoid medications that previously triggered SJS or drugs similar to them.

Outlook and Prognosis of Stevens-Johnson Syndrome

The outlook for individuals affected by Stevens-Johnson syndrome (SJS) can vary widely, with recovery timelines ranging from weeks to months, depending on the severity of symptoms. While some may experience relatively quick skin regrowth, others may require more extended periods for recovery, especially if symptoms are severe. However, long-term complications and reactions may persist, including:

Skin Issues: Dryness, itching, and changes in skin color can occur.
Eye Complications: Chronic swelling, dryness, irritation, vision difficulties, and sensitivity to light (photophobia) may persist.
Excessive Sweating: Some individuals may experience increased sweating.
Respiratory Problems: Lung damage, chronic obstructive pulmonary disease (COPD), and asthma can develop as long-term consequences.
Nail and Hair Changes: Nail loss or deformity, as well as hair loss (alopecia), may occur.
Mucous Membrane Dryness: Dryness of mucous membranes can lead to difficulties with urination.
Chronic Fatigue: Chronic fatigue syndrome may develop post-SJS.
Altered Sense of Taste: Difficulties with the sense of taste may be experienced.

It’s important to note that SJS may reoccur if exposed to the same triggering medication. Second episodes tend to be more severe than the initial occurrence.

Furthermore, SJS can have severe outcomes, with a 10% fatality rate for patients and a 30% fatality rate for those with toxic epidermal necrolysis (TEN). The primary causes of death are often related to complications such as sepsis, acute respiratory distress syndrome, and multiple organ failure.

FAQs

Severity of SJS: While Stevens-Johnson syndrome is rarely fatal, it necessitates hospital treatment, often in intensive care or a burn unit. Most individuals recover, although some may experience long-term complications affecting the skin, eyes, and lungs.
Progression of SJS: SJS typically begins with flu-like symptoms and eye irritation, followed by the development of a rash on the skin and mucous membranes within 1 to 3 days. With prompt treatment, skin healing usually occurs within 2 to 3 weeks. Notably, SJS can manifest up to 2 weeks after discontinuing the triggering medication.
Conditions Mistaken for SJS: Certain autoimmune diseases and allergic reactions, such as erythema multiforme major, can resemble SJS. It’s essential for healthcare providers to conduct thorough evaluations to differentiate between these conditions accurately.

Given the potential for significant long-term effects and the risk of recurrence, individuals affected by SJS require ongoing medical monitoring and support to manage symptoms and mitigate risks effectively.

Sources

UpToDate: “Stevens-Johnson syndrome and toxic epidermal necrolysis: Pathogenesis, clinical manifestations, and diagnosis,” “Stevens-Johnson syndrome and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae.”
WebMD: “Stevens-Johnson syndrome.”
FDA: “FDA Warns of Rare Acetaminophen Risk,” “Information for Healthcare Professionals: Dangerous or Even Fatal Skin Reactions – Carbamazepine (marketed as Carbatrol, Equetro, Tegretol, and generics).”
Fritsch, P. (2000). American Journal of Clinical Dermatology. November-December 2000.
Mayo Clinic: “Stevens-Johnson syndrome.”
National Health Service (U.K.): “Stevens-Johnson Syndrome.”
American Journal of Clinical Dermatology: “Differential diagnosis of severe cutaneous drug eruptions.”
Levi, N. (2009). American Journal of Pediatrics. January 2009.
Medscape: “Stevens-Johnson Syndrome Treatment & Management.”
Merck Manual: “Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN).”
Cutaneous Drug Eruptions: “Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.”
Cleveland Clinic: “Stevens-Johnson Syndrome,” “Graft vs. Host Disease,” “Sepsis,” “Organ Failure,” “Erythema Multiforme.”
National Organization for Rare Diseases (NORD): “Stevens Johnson Syndrome.”
Tartarone, A. (2010). Therapeutic Drug Monitoring. December 2010.
StatPearls: “Toxic Epidermal Necrolysis.”

SJS Stevens-Johnson Syndrome